Journal
NEUROEPIDEMIOLOGY
Volume 42, Issue 2, Pages 81-89Publisher
KARGER
DOI: 10.1159/000356351
Keywords
Essential tremor; Disease; Classification; Pathology; Genetics; Clinical features; Heterogeneity; Neurodegeneration
Funding
- National Institutes of Health [R01 NS042859, R01 NS39422, R01 NS073872]
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There is accumulating evidence that the entity referred to as 'essential tremor' (ET) is not a single disease. It may be a family of diseases better referred to as 'the ETs'. This review will summarize the following evidence: (1) the presence of etiological heterogeneity; (2) the heterogeneity of findings in postmortem studies, thus suggesting several diseases; (3) the recent discussion that age of onset may be an important marker of disease heterogeneity; (4) the clinical expansion of the concept of ET in recent years to include a broader range of tremor phenomenology, other motor features (gait ataxia), other involuntary movements (dystonia), and nonmotor features (cognitive problems, psychiatric problems), some of which could be primary; (5) the heterogeneity of pharmacological response profiles and clinical progression, and (6) the association of ET with Parkinson's disease, Alzheimer's disease, and possibly progressive supranuclear palsy, with the possibility that some ET patients are more predisposed to develop one of these. (C) 2013 S. Karger AG, Basel
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